Eisenmenger's Syndrome

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Eisenmenger's Syndrome

In this article

Eisenmenger's syndrome is defined as obstructive pulmonary vascular disease that develops as a consequence of a large pre-existing left-to-right shunt causing pulmonary artery pressures to increase and approach systemic levels, such that the direction of blood flow then becomes bi-directional or right-to-left.[1]

The frequency of pulmonary hypertension and development of reversed shunting vary depending on the specific heart defect and operative interventions. Early development of Eisenmenger's syndrome is more commonly associated with persistent truncus arteriosus and unrestricted pulmonary blood flow, common atrioventricular canal, ventricular septal defect (VSD), patent ductus arteriosus (PDA) and transposition of the great arteries.

The high pulmonary vascular resistance is usually established by age 2 years and can sometimes be present from birth. It is less common and occurs later in life in patients with a large secundum atrial septal defect (ASD).

Epidemiology

The frequency of pulmonary hypertension and the development of reversed shunting vary depending on the specific heart defect and operative interventions.

50% of infants with a large, non-restrictive VSD or PDA develop pulmonary hypertension by early childhood.

10% of patients with a large secundum ASD progress to pulmonary hypertension but usually not until after the third decade of life.

Dyspnoea, fatigue, syncope; exercise intolerance (dyspnoea and fatigue) is proportional to the degree of hypoxaemia or cyanosis.

Chest pain.

Haemoptysis.

Examination reveals:

Cyanosis, clubbing and plethora.

Right ventricular heave with palpable, loud pulmonary component of the second heart sound.

Loud second heart sound with a narrow split.

Ejection systolic murmur audible along the left sternal border.

Graham Steell murmur: a diastolic murmur audible along the left sternal border due to functional incompetence of the pulmonary valve in patients with pulmonary hypertension. The Graham Steell murmur is a high-pitched, decrescendo murmur, loudest during inspiration.

Flying on commercial airline flights can be safely performed with stable patients and SaO2 on room air >85%.

Management

Treat heart failure and arrhythmias.

Calcium-channel blockers (may increase the right-to-left shunt), antiplatelet agents and anticoagulants have not been shown to be beneficial and may cause further complications - eg, hypotension, worsening cyanosis, increased hyperuricaemia or haemorrhage.

Prevention of infective endocarditis:

The National Institute for Health and Care Excellence (NICE) recommends that if a person at risk of infective endocarditis is receiving antimicrobial therapy because they are undergoing a gastrointestinal or genitourinary procedure at a site where there is a suspected infection, the person should receive an antibiotic that covers organisms that cause infective endocarditis.

Any episodes of infection in people at risk of infective endocarditis should be investigated and treated promptly to reduce the risk of endocarditis developing.

Patients with significant polycythaemia may be helped by repeated venesection and volume replacement. Phlebotomies are required only when hyperviscosity of the blood is evident, usually when the haematocrit is above 65%.[3]

Chronic use of oxygen or pulmonary vasodilators is controversial and under investigation.

With continued improvements in the diagnosis, pre-operative management, refinement of surgical techniques and postoperative management strategies, patients with Eisenmenger's syndrome might be amenable to, and benefit from, repair in the modern era.[4]

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